CORRESPONDENCE

Correlation Between RetinalAbnormalities and IntracranialAbnormalities in the Shaken BabySyndrome

EDITOR:

I READ WITH KEEN INTEREST THE ARTICLE BY MORAD AND

associates (Am J Ophthalmol 134:354–359, 2002) on thecorrelation between retinal abnormalities and intracranialabnormalities in the shaken baby syndrome. It is aninteresting piece of work, and I would like to extend myappreciation to all of the authors. It was interesting to notethat the severity of the retinal and intracranial injuries arecorrelated. Child abuse is almost always underdiagnosedand underreported. In one study, more than 75% of allchild abuse cases presenting to an emergency departmentwere reported to be missed.1 Therefore, we all need to beextra vigilant and sensitive to any child presenting withunusual symptoms and signs. However, not all such un-usual cases could be of child abuse, for example, hemoph-agocytic lymphohistiocytosis. Retinal and intracranialhemorrhages are the major neuropathologic findings inchildren with this disease. Patients who present withocular and central nervous system findings may havesymptoms that mimic those of inflicted injury. Thesechildren are at risk, therefore, for misdiagnosis as victims ofchild abuse. Such an error causes not only unnecessaryadditional trauma to the family but also, more importantly,a delay in initiating effective therapy.2

Ocular involvement is also common in glutaric aciduria.Complete ophthalmologic evaluation is recommended inall patients suspected of having this rare disease. Intrareti-nal hemorrhages due to glutaric aciduria could be misin-terpreted as resulting from child abuse, and it is importantto include this disorder with the differential diagnosis ofchild abuse.3 There have been controversies with respectto the fracture of the ribs, which could point toward childabuse, as the caregiver might have tried to suffocate thechild. However, we must not forget that the same couldpossibly happen when a child is being resuscitated. Besides,there are other reasons of retinal hemorrhages in children.It would be interesting to know if they were duly ruled out.

The authors have mentioned that some patients died.Could the authors kindly mention the most common causeof death as per the autopsies?

AKASH RAJ, MD, DNB, DRCO, FRCS

London, England

REFERENCES

1. Kunen S, Hume P, Perret JN, Mandry CV, Patterson TR.Underdiagnosis of child abuse in emergency departments.Acad Emerg Med 2003;10:546.

2. Rooms L, Fitzgerald N, McClain KL. Hemophagocytic lym-phohistiocytosis masquerading as child abuse: Presentation ofthree cases and review of central nervous system findings inhemophagocytic lymphohistiocytosis. Paediatrics 2003;111(5Pt 1):636–640.

3. Kafil-Hussain NA, Monavari A, Bowell R, Thornton P,Naughten E, O’Keefe M. Ocular findings in glutaric aciduriatype 1. J Pediatr Ophthalmol Strabismus 2000;37:289–293.

AUTHOR REPLY

WE THANK DR. RAJ FOR HIS INTEREST IN OUR ARTICLE. WE

certainly agree that child abuse is often underdiagnosedand underrecognized. Rarely, other conditions presentsome of the same manifestations as the shaken babysyndrome (SBS)/abusive head injury, which may result inconfusion if not misdiagnosis.

Before our 2002 report, a review of reported cases ofhemophagocytic lymphohistiocytosis reveals no cases inwhich retinal hemorrhages appeared in the absence ofretinal infiltrates and/or optic nerve infiltration/swelling.In addition, patients almost always present with hepato-splenomegaly, coagulopathy, and other systemic abnormal-ities, which would not likely be confused with abusivehead injury. The paper by Rooms and coworkers, publishedin 2003 and cited by Dr. Raj, suggests that this disordermay, in extraordinary cases, be confused with SBS. How-ever, in two of the three cases (cases 2 and 3), it appearslikely that the children were victims of both nonaccidentalhead injury and hemophagacytic lymphohistiocytosis. Al-though not included as authors on the paper, the treatingphysicians for case 2 believe strongly that SBS was acomorbid condition (Karen Hansen, personal communica-tion). Case 3 also had a probable fracture due to abuse.This leaves only case 1 in which the patient had a retinalappearance that should not be confused with SBS: perivas-cular hemorrhages and retinal necrosis. Retinal necrosis, tomy knowledge, has never been reported in SBS. As ourpatients all presented before the 2003 article, none had

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